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Human Metabolome Database Version 3.5

Showing metabocard for Succinic acid (HMDB00254)

• Identification
• Taxonomy
• Ontology
• Physical properties
• Spectra
• Biological properties
• Concentrations
• Links
• References

enzymes (44)

• Blood
• Urine

Record Information
Version	3.5
Creation Date	2005-11-16 08:48:42 -0700
Update Date	2013-05-29 13:25:16 -0600
HMDB ID	HMDB00254
Secondary Accession Numbers	None
Metabolite Identification
Common Name	Succinic acid
Description	Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825 ) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021 ).
Structure	Download: MOL | SDF | SMILES | InChI Display: 2D Structure | 3D Structure
Synonyms	1,2-Ethanedicarboxylate 1,2-Ethanedicarboxylic acid 1,4-Butanedioate 1,4-Butanedioic acid Amber acid Asuccin Dihydrofumarate Dihydrofumaric acid Katasuccin Succinate Wormwood acid
Chemical Formula	C4H6O4
Average Molecular Weight	118.088
Monoisotopic Molecular Weight	118.02660868
IUPAC Name	butanedioic acid
Traditional IUPAC Name	succinic acid
CAS Registry Number	110-15-6
SMILES	OC(=O)CCC(O)=O
InChI Identifier	InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
InChI Key	KDYFGRWQOYBRFD-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom	Organic Compounds
Super Class	Organic Acids and Derivatives
Class	Carboxylic Acids and Derivatives
Sub Class	Dicarboxylic Acids and Derivatives
Other Descriptors	Aliphatic Acyclic Compounds Dicarboxylic acids(Lipidmaps) Fatty Acids and Conjugates alpha,omega-dicarboxylic acid(ChEBI)
Substituents	Carboxylic Acid
Direct Parent	Dicarboxylic Acids and Derivatives
Ontology
Status	Detected and Quantified
Origin	Endogenous Microbial
Biofunction	Component of Arginine and proline metabolism Component of Butanoate metabolism Component of C5-Branched dibasic acid metabolism Component of Glutamate metabolism Component of Propanoate metabolism DNA component
Application	Not Available
Cellular locations	Extracellular Mitochondria Endoplasmic reticulum Peroxisome
Physical Properties
State	Solid
Experimental Properties	Property Value Reference Melting Point 185 - 188 °C Not Available Boiling Point Not Available Not Available Water Solubility 83.2 mg/mL Not Available LogP -0.59 HANSCH,C ET AL. (1995)
Predicted Properties	Property Value Source Water Solubility 211 g/L ALOGPS LogP -0.53 ALOGPS LogP -0.4 ChemAxon LogS 0.25 ALOGPS pKa (strongest acidic) 3.55 ChemAxon Hydrogen Acceptor Count 4 ChemAxon Hydrogen Donor Count 2 ChemAxon Polar Surface Area 74.6 A2 ChemAxon Rotatable Bond Count 3 ChemAxon Refractivity 23.54 ChemAxon Polarizability 10.16 ChemAxon Formal Charge 0 ChemAxon Physiological Charge -2 ChemAxon
Spectra
	Gas-MS Spectrum 13C NMR Spectrum 1H NMR Spectrum MS/MS Spectrum Quattro_QQQ 10 MS/MS Spectrum Quattro_QQQ 25 MS/MS Spectrum Quattro_QQQ 40 MS/MS Spectrum EI-B (Unknown) MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) MS/MS Spectrum LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50 MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies ) [1H,1H] 2D NMR Spectrum [1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations	Extracellular Mitochondria Endoplasmic reticulum Peroxisome
Biofluid Locations	Blood Cerebrospinal Fluid (CSF) Saliva Urine
Tissue Location	Muscle Skeletal Muscle Fibroblasts Pancreas Placenta Kidney Liver Brain Prostate Adipose Tissue Spleen
Pathways	Name SMPDB Link KEGG Link Citric Acid Cycle SMP00057 map00020 Glutamate Metabolism SMP00072 map00250 Mitochondrial Electron Transport Chain SMP00355 map00190 Phytanic Acid Peroxisomal Oxidation SMP00450 Not Available Carnitine Synthesis SMP00465 Not Available
Normal Concentrations
	Biofluid Status Value Age Sex Condition Reference Blood Detected and Quantified 16.0 (0.00-32.0) uM Adult (>18 years old) Both Normal 11067816 Blood Detected and Quantified 23.5 uM Adult (>18 years old) Not Specified Normal 21359215 Blood Detected and Quantified 8.8 +/- 2.7 uM Adult (>18 years old) Both Normal Geigy Scient... Cerebrospinal Fluid (CSF) Detected and Quantified 47.2 uM Adult (>18 years old) Both Normal 18953024 Cerebrospinal Fluid (CSF) Detected and Quantified 19 uM Adult (>18 years old) Both Normal 7729054 Cerebrospinal Fluid (CSF) Detected and Quantified 150.0 +/- 110.0 uM Elderly (>65 years old) Both Normal 9693263 Cerebrospinal Fluid (CSF) Detected and Quantified 3 +/- 2 uM Not Specified Both Normal 18502700 Cerebrospinal Fluid (CSF) Detected and Quantified 28.5 (24-33) uM Adult (>18 years old) Both Normal Geigy Scient... Cerebrospinal Fluid (CSF) Detected and Quantified 3.0 +/- 2.0 uM Adult (>18 years old) Both Normal 8412012 Saliva Detected and Quantified 2260 (60.0-4460) uM Adult (>18 years old) Both Normal 12097436 Saliva Detected and Quantified 4-145 uM Adult (>18 years old) Male normal 19259987 Saliva Detected and Quantified 1-33 uM Adult (>18 years old) Male normal 19259987 Urine Detected but not Quantified Not Applicable Adult (>18 years old) Male Normal 22932811 Urine Detected and Quantified 4.7 (1.1-14.5) umol/mmol creatinine Adult (>18 years old) Both Normal Bouatra, S. ... Urine Detected and Quantified 12.60 +/- 12.13 umol/mmol creatinine Infant (0-1 year old) Both Normal 2026685 Urine Detected and Quantified 14.48 +/- 3.20 umol/mmol creatinine Children (1-13 year old) Both Normal 10731506 Urine Detected but not Quantified Not Applicable Adult (>18 years old) Both Normal Bouatra, S. ... Urine Detected and Quantified 7.7 (1.9-20.0) umol/mmol creatinine Adolescent (13-18 years old) Both Normal 8087979 Urine Detected and Quantified 3.8 (1.25-6.7) umol/mmol creatinine Adult (>18 years old) Both Normal Geigy Scient... Urine Detected but not Quantified Not Applicable Adult (>18 years old) Both Normal 21389975 Urine Detected and Quantified 2.10 umol/mmol creatinine Adult (>18 years old) Both Normal Shaykhutdino... Urine Detected and Quantified 9.9 +/- 5.0 umol/mmol creatinine Adult (>18 years old) Male Normal 17474139 Urine Detected and Quantified 14.4 +/- 4.9 umol/mmol creatinine Adult (>18 years old) Female Normal 17474139 Urine Detected and Quantified 7.5 (0.5-16) umol/mmol creatinine Adult (>18 years old) Both Normal 2702744 Urine Detected and Quantified 6.0 (0.3-33.3) umol/mmol creatinine Adult (>18 years old) Both Normal Bouatra, S. ... Urine Detected but not Quantified Not Applicable Adult (>18 years old) Male Normal 16939360 Urine Detected but not Quantified Not Applicable Adult (>18 years old) Male Normal 16939360 Urine Detected and Quantified 5.6 +/- 3.8 umol/mmol creatinine Adult (>18 years old) Both Normal 15992788 Urine Detected and Quantified 197.2 (29.4-486.2) umol/mmol creatinine Newborn (0-30 days old) Both Normal 8087979 Urine Detected and Quantified 185.4 (6.0-342.6) umol/mmol creatinine Infant (0-1 year old) Both Normal 8087979 Urine Detected and Quantified 11.6 (4.0-27.3) umol/mmol creatinine Children (1-13 year old) Both Normal 8087979 Urine Detected and Quantified 8.25 (0.5-16.0) umol/mmol creatinine Adult (>18 years old) Both Normal 11067816 Urine Detected and Quantified 1.3 umol/mmol creatinine Adult (>18 years old) Both Normal 21388201 Urine Detected and Quantified 6.2 (2.5-13.5) umol/mmol creatinine Adult (>18 years old) Both Normal Bouatra, S. ...
Abnormal Concentrations
	Biofluid Status Value Age Sex Condition Reference Cerebrospinal Fluid (CSF) Detected and Quantified 19.0 (0.0-38.0) uM Adult (>18 years old) Both Canavan Disease 7729054 Cerebrospinal Fluid (CSF) Detected and Quantified 55.0 (19.0-91.0) uM Adult (>18 years old) Both Alzheimer's disease 9693263 Urine Detected and Quantified 43.35 +/- 25.08 umol/mmol creatinine Children (1-13 year old) Both Severely malnourished children 10731506 Urine Detected and Quantified 9.0 +/- 6.0 umol/mmol creatinine Adult (>18 years old) Both Lung cancer 18953024
Associated Disorders and Diseases
Disease References	Canavan disease Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. Pubmed: 7729054 Alzheimer's disease Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Lung Cancer Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024
Associated OMIM IDs	271900 (Canavan disease) 104300 (Alzheimer's disease) 211980 (Lung Cancer)
External Links
DrugBank ID	DB00139
DrugBank Metabolite ID	Not Available
Phenol Explorer Compound ID	Not Available
Phenol Explorer Metabolite ID	Not Available
FoodDB ID	FDB001931
KNApSAcK ID	C00001205
Chemspider ID	1078
KEGG Compound ID	C00042
BioCyc ID	SUC
BiGG ID	33633
Wikipedia Link	Succinic acid
NuGOwiki Link	HMDB00254
Metagene Link	HMDB00254
METLIN ID	114
PubChem Compound	1110
PDB ID	SIN
ChEBI ID	15741
References
Synthesis Reference	Berglund, Kris Arvid; Andersson, Christian; Rova, Ulrika. Process for the production of succinic acid. PCT Int. Appl. (2007), 30pp.
Material Safety Data Sheet (MSDS)	Download (PDF)
General References	Magera MJ, Helgeson JK, Matern D, Rinaldo P: Methylmalonic acid measured in plasma and urine by stable-isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2000 Nov;46(11):1804-10. Pubmed: 11067816 Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979 Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. Pubmed: 9693263 Zhang TM, Sener A, Malaisse WJ: Hydrolysis of succinic acid dimethyl ester in rat pancreatic islets. Biochem Mol Med. 1995 Aug;55(2):131-7. Pubmed: 7582870 Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. Pubmed: 7729054 Groenen PM, Engelke UF, Wevers RA, Hendriks JC, Eskes TK, Merkus HM, Steegers-Theunissen RP: High-resolution 1H NMR spectroscopy of amniotic fluids from spina bifida fetuses and controls. Eur J Obstet Gynecol Reprod Biol. 2004 Jan 15;112(1):16-23. Pubmed: 14687733 Meijer-Severs GJ, van Santen E: Short-chain fatty acids and succinate in feces of healthy human volunteers and their correlation with anaerobe cultural counts. Scand J Gastroenterol. 1987 Aug;22(6):672-6. Pubmed: 3659829 Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. Pubmed: 12097436 Ren LC, Huang XY, Long JH: [Effects of succinic acid on the function of in vitro cultured human fibroblasts] Zhonghua Shao Shang Za Zhi. 2004 Feb;20(1):34-6. Pubmed: 15059451 Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. Pubmed: 8412012 Wevers RA, Engelke U, Heerschap A: High-resolution 1H-NMR spectroscopy of blood plasma for metabolic studies. Clin Chem. 1994 Jul;40(7 Pt 1):1245-50. Pubmed: 8013094 Borenstein DG, Gibbs CA, Jacobs RP: Gas-liquid chromatographic analysis of synovial fluid: volatile short-chain fatty acids in septic arthritis. Ann Rheum Dis. 1983 Aug;42(4):362-7. Pubmed: 6882030 Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. Pubmed: 1126459 Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 Briere JJ, Favier J, El Ghouzzi V, Djouadi F, Benit P, Gimenez AP, Rustin P: Succinate dehydrogenase deficiency in human. Cell Mol Life Sci. 2005 Oct;62(19-20):2317-24. Pubmed: 16143825 Rustin P, Rotig A: Inborn errors of complex II--unusual human mitochondrial diseases. Biochim Biophys Acta. 2002 Jan 17;1553(1-2):117-22. Pubmed: 11803021

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Enzymes
Name: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial Reactions: Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details Gene Name: SDHC Uniprot ID: Q99643 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial Reactions: Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details Gene Name: SDHA Uniprot ID: P31040 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial Reactions: Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details Gene Name: SDHD Uniprot ID: O14521 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Reactions: Succinic acid + Coenzyme Q10 unknown Fumaric acid + QH(2) details Ubiquinone-2 + Succinic acid unknown QH2 + Fumaric acid details Gene Name: SDHB Uniprot ID: P21912 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinate-semialdehyde dehydrogenase, mitochondrial Reactions: Succinic acid semialdehyde + NAD + Water unknown Succinic acid + NADH details Succinic acid semialdehyde + NAD + Water unknown Succinic acid + NADH + Hydrogen Ion details Gene Name: ALDH5A1 Uniprot ID: P51649 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phytanoyl-CoA dioxygenase, peroxisomal Reactions: Phytanoyl-CoA + Oxoglutaric acid + Oxygen unknown 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2) details Gene Name: PHYH Uniprot ID: O14832 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 4-hydroxylase subunit alpha-2 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details L-Proline + Oxoglutaric acid + Oxygen unknown Hydroxyproline + Succinic acid + Carbon dioxide details Gene Name: P4HA2 Uniprot ID: O15460 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 4-hydroxylase subunit alpha-1 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details L-Proline + Oxoglutaric acid + Oxygen unknown Hydroxyproline + Succinic acid + Carbon dioxide details Gene Name: P4HA1 Uniprot ID: P13674 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Gamma-butyrobetaine dioxygenase Reactions: 4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + CO(2) details 4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen unknown L-Carnitine + Succinic acid + Carbon dioxide details Gene Name: BBOX1 Uniprot ID: O75936 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1 Reactions: L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details Gene Name: PLOD1 Uniprot ID: Q02809 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2 Reactions: L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details Gene Name: PLOD2 Uniprot ID: O00469 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 Reactions: L-lysine-[procollagen] + Oxoglutaric acid + Oxygen unknown (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2) details Protein lysine + Oxoglutaric acid + Oxygen unknown Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water details Gene Name: PLOD3 Uniprot ID: O60568 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial Reactions: Succinyl-CoA + a 3-oxo acid unknown Succinic acid + a 3-oxoacyl-CoA details Succinyl-CoA + Acetoacetic acid unknown Succinic acid + Acetoacetyl-CoA details Gene Name: OXCT1 Uniprot ID: P55809 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial Reactions: Succinyl-CoA + a 3-oxo acid unknown Succinic acid + a 3-oxoacyl-CoA details Succinyl-CoA + Acetoacetic acid unknown Succinic acid + Acetoacetyl-CoA details Gene Name: OXCT2 Uniprot ID: Q9BYC2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial Reactions: Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details Adenosine triphosphate + Succinic acid + Coenzyme A unknown ADP + Phosphoric acid + Succinyl-CoA details Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details Gene Name: SUCLG1 Uniprot ID: P53597 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial Reactions: Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details Gene Name: SUCLG2 Uniprot ID: Q96I99 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial Reactions: Adenosine triphosphate + Succinic acid + Coenzyme A unknown ADP + Phosphoric acid + Succinyl-CoA details Guanosine triphosphate + Succinic acid + Coenzyme A unknown Guanosine diphosphate + Phosphoric acid + Succinyl-CoA details Inosine triphosphate + Succinic acid + Coenzyme A unknown IDP + Phosphoric acid + Succinyl-CoA details Gene Name: SUCLA2 Uniprot ID: Q9P2R7 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aspartyl/asparaginyl beta-hydroxylase Reactions: Peptide L-aspartate + Oxoglutaric acid + Oxygen unknown peptide 3-hydroxy-L-aspartate + Succinic acid + CO(2) details Gene Name: ASPH Uniprot ID: Q12797 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Hypoxia-inducible factor 1-alpha inhibitor Reactions: Hypoxia-inducible factor-L-asparagine + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-(3S)-3-hydroxy-L-asparagine + Succinic acid + CO(2) details Ankyrin-repeat-L-histidine + Oxoglutaric acid + Oxygen unknown ankyrin-repeat-(3S)-3-hydroxy-L-histidine + Succinic acid + CO(2) details Gene Name: HIF1AN Uniprot ID: Q9NWT6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Mitochondrial dicarboxylate carrier Reactions: Not Available Gene Name: SLC25A10 Uniprot ID: Q9UBX3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Solute carrier family 13 member 3 Reactions: Not Available Gene Name: SLC13A3 Uniprot ID: Q8WWT9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: 17-beta-hydroxysteroid dehydrogenase type 6 Reactions: Not Available Gene Name: HSD17B6 Uniprot ID: O14756 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Solute carrier family 13 member 1 Reactions: Not Available Gene Name: SLC13A1 Uniprot ID: Q9BZW2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Solute carrier family 13 member 2 Reactions: Not Available Gene Name: SLC13A2 Uniprot ID: Q13183 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Trimethyllysine dioxygenase, mitochondrial Reactions: N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown 3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + Succinic acid + CO(2) details N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxide details Gene Name: TMLHE Uniprot ID: Q9NVH6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alpha-ketoglutarate-dependent dioxygenase alkB homolog 2 Reactions: DNA-base-CH(3) + Oxoglutaric acid + Oxygen unknown DNA-base + Formaldehyde + Succinic acid + CO(2) details Gene Name: ALKBH2 Uniprot ID: Q6NS38 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 3-hydroxylase 1 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details Gene Name: LEPRE1 Uniprot ID: Q32P28 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 3-hydroxylase 2 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details Gene Name: LEPREL1 Uniprot ID: Q8IVL5 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 3-hydroxylase 3 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details Gene Name: LEPREL2 Uniprot ID: Q8IVL6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Prolyl 4-hydroxylase subunit alpha-3 Reactions: L-proline-[procollagen] + Oxoglutaric acid + Oxygen unknown trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details L-Proline + Oxoglutaric acid + Oxygen unknown Hydroxyproline + Succinic acid + Carbon dioxide details Gene Name: P4HA3 Uniprot ID: Q7Z4N8 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Lysine-specific demethylase 2A Reactions: Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N6,N6-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown Protein lysine + Succinic acid + Formaldehyde + Carbon dioxide details Gene Name: KDM2A Uniprot ID: Q9Y2K7 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Lysine-specific demethylase 2B Reactions: Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N6,N6-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown Protein lysine + Succinic acid + Formaldehyde + Carbon dioxide details Gene Name: KDM2B Uniprot ID: Q8NHM5 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Egl nine homolog 1 Reactions: Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details Gene Name: EGLN1 Uniprot ID: Q9GZT9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Egl nine homolog 2 Reactions: Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details Gene Name: EGLN2 Uniprot ID: Q96KS0 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Egl nine homolog 3 Reactions: Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen unknown hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2) details Gene Name: EGLN3 Uniprot ID: Q9H6Z9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Transmembrane prolyl 4-hydroxylase Reactions: An HIF alpha chain L-proline + Oxoglutaric acid + Oxygen unknown An HIF alpha chain trans-4-hydroxy-L-proline + Succinic acid + CO(2) details Gene Name: P4HTM Uniprot ID: Q9NXG6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alkylated DNA repair protein alkB homolog 1 Reactions: DNA-base-CH(3) + Oxoglutaric acid + Oxygen unknown DNA-base + Formaldehyde + Succinic acid + CO(2) details Gene Name: ALKBH1 Uniprot ID: Q13686 Protein Sequence: FASTA
Name: Lysine-specific demethylase 8 Reactions: Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details Gene Name: KDM8 Uniprot ID: Q8N371 Protein Sequence: FASTA
Name: Bifunctional lysine-specific demethylase and histidyl-hydroxylase MINA Reactions: L-histidine-[60S ribosomal protein L27a] + Oxoglutaric acid + Oxygen unknown (3S)-3-hydroxy-L-histidine-[60S ribosomal protein L27a] + Succinic acid + CO(2) details Gene Name: MINA Uniprot ID: Q8IUF8 Protein Sequence: FASTA
Name: Bifunctional lysine-specific demethylase and histidyl-hydroxylase NO66 Reactions: Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details L-histidine-[60S ribosomal protein L8] + Oxoglutaric acid + Oxygen unknown (3S)-3-hydroxy-L-histidine-[60S ribosomal protein L8] + Succinic acid + CO(2) details Gene Name: NO66 Uniprot ID: Q9H6W3 Protein Sequence: FASTA
Name: Histone lysine demethylase PHF8 Reactions: Protein N(6),N(6)-dimethyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein N(6)-methyl-L-lysine + Succinic acid + Formaldehyde + CO(2) details Protein N(6)-methyl-L-lysine + Oxoglutaric acid + Oxygen unknown protein L-lysine + Succinic acid + Formaldehyde + CO(2) details Gene Name: PHF8 Uniprot ID: Q9UPP1 Protein Sequence: FASTA
Name: Methylcytosine dioxygenase TET1 Reactions: DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details Gene Name: TET1 Uniprot ID: Q8NFU7 Protein Sequence: FASTA
Name: Methylcytosine dioxygenase TET2 Reactions: DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details Gene Name: TET2 Uniprot ID: Q6N021 Protein Sequence: FASTA
Name: Methylcytosine dioxygenase TET3 Reactions: DNA 5-methylcytosine + Oxoglutaric acid + Oxygen unknown DNA 5-hydroxymethylcytosine + Succinic acid + CO(2) details Gene Name: TET3 Uniprot ID: O43151 Protein Sequence: FASTA

This project is supported by Genome Alberta & Genome Canada, a not-for-profit organization that is leading Canada's national genomics strategy with $600 million in funding from the federal government.